Abstract: Publication date: Available online 2 July 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Patrycja Chrząszcz, Kamil Derbisz, Krzysztof Suszyński, Jan Miodoński, Robert Trybulski, Joanna Lewin-Kowalik, Wiesław Marcol Understanding the pathomechanisms behind peripheral nerve damage and learning the course of regeneration seem to be crucial for selecting the appropriate methods of treatment. Autografts are currently the gold standard procedure in nerve reconstruction. However, due to the frequency of complications resulting from autografting and a desire to create a better environment for the regeneration of the damaged nerve, artificial conduits have become an approved alternative treatment method. The aim of this mini-review is to present the nerve scaffolds that have been applied in clinical practice to date, and the potential directions of developments in nerve conduit bioengineering.Articles regarding construction and characterization of nerve conduits were used as the theoretical background. All papers, available in PubMed database since 2000, presenting results of application of artificial nerve conduits in clinical trials were included into this mini-review.Fourteen studies including ≤10 patients and 10 trials conducted on>10 patients were analyzed as well as 24 papers focused on artificial nerve conduits per se. Taking into consideration the experiences of the authors investigating nerve conduits in clinical trials, it is essential to point out the emergence of bioresorbable scaffolds, which in the future may significantly change the treatment of peripheral nerve injuries. Also worth mentioning among the advanced conduits are hybrid conduits, which combine several modifications of a synthetic material to provide the optimal regeneration of a damaged nerve.

Abstract: Publication date: Available online 2 July 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Nicola Montano, Vito Stifano, Fabio Papacci, Edoardo Mazzucchi, Eduardo Fernandez We reported the results of minimally invasive spinal decompression (MISD) in patients with degenerative spondylolisthesis (DS) associated with lumbar spinal stenosis (LSS) and performed a literature review in order to evaluate the clinical and radiological outcomes, the complications and reoperation rate of MISD procedures in these patients.Data of 28 patients submitted to MISD for DS associated to LSS were reviewed. We evaluated the Visual Analogue Scale (VAS) both for low back pain (LBP) and legs pain, the Oswestry Disability Index (ODI) and the degree of the slippage. A PubMed search of the English literature was conducted. Only papers with more than 10 patients and reporting explicitly data of patients with DS were included in the analysis. We found a statistically significant improvement of LBP, legs pain and ODI in our series. The degree of slippage was stable at follow-up (FU) with no need of reoperation. No major complications occurred. In our literature review, we were able to analyze the differences in ODI in 156 patients and the differences in Japanese Orthopedic Association (JOA) score in 218 patients. We observed a statistically significant improvement of ODI and JOA score at FU compared to pre-operative. The percentage of slippage, evaluated in 283 patients, was unchanged at FU compared to pre-operative. The overall complication rate was 1.6%. The overall reoperation rate was 4.5%.MISD procedures are safe and effective in patients with DS associated to LSS and are associated to low morbidity and significant improvement of disability without progression of slippage.

Abstract: Publication date: Available online 30 June 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Eric D. Goldstein, Anteneh M. Feyissa IntroductionGliomas are commonly associated with the development of epilepsy; in some cases the two conditions share common pathogenic mechanisms and may influence each other. Brain tumor related-epilepsy (BTRE) complicates the clinical management of gliomas and can substantially affect daily life.State of the artThe incidence of seizures is high in patients with slow growing tumors located in the frontotemporal regions. However, recent studies suggest that epileptogenesis may be more associated with tumor molecular genetic markers than tumor grade or location. Although the exact mechanism of epileptogenesis in glioma is incompletely understood, glutamate-induced excitotoxicity and disruption of intracellular communication have garnered the most attention.Clinical managementManagement of BTRE requires a multidisciplinary approach involving the use of antiepileptic drugs (AEDs), surgery aided by electrocorticography, and adjuvant chemoradiation.Future directionsInsight into the mechanisms of glioma growth and epileptogenesis is essential to identify new treatment targets and to develop effective treatment for both conditions. Selecting AEDs tailored to act against known tumor molecular markers involved in the epileptogenesis could enhance treatment value and help inform individualized medicine in BRTE.

Abstract: Publication date: Available online 13 June 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): J. Jędrzejczak, M. Mazurkiewicz-Bełdzińska, M. Szmuda, B. Majkowska-Zwolińska, B. Steinborn, D. Ryglewicz, R. Owczuk, A. Bartkowska-Śniatkowska, E. Widera, K. Rejdak, M. Siemiński, E. Nagańska IntroductionThe Working Group was established at the initiative of the General Board of the Polish Society of Epileptology (PSE) to develop an expert position on the treatment of convulsive status epilepticus (SE) in adults and children in Poland. Generalized convulsive SE is the most common form and also represents the greatest threat to life, highlighting the importance of the choice of appropriate therapeutic treatment.Aim of guidelineWe present the therapeutic options separately for treatment during the early preclinical (>5–30 min), established (30–60 min), and refractory (>60 min) SE phases. This division is based on time and response to AEDs, and indicates a practical approach based on pathophysiological data.ResultsBenzodiazepines (BZD) are the first-line drugs. In cases of ineffective first-line treatment and persistence of the seizure, the use of second-line treatment: phenytoin, valproic acid or phenobarbital is required. SE that persists after the administration of benzodiazepines and phenytoin or another second-line AED at appropriate doses is defined as refractory and drug resistant and requires treatment in the intensive care unit (ICU). EEG monitoring is essential during therapy at this stage. Anesthesia is typically continued for an initial period of 24 h followed by a slow reversal and is re-established if seizures recur. Anesthesia is usually administered either to the level of the “burst suppression pattern” or to obtain the “EEG suppression” pattern.ConclusionsExperts agree that close and early cooperation with a neurologist and anesthetist aiming to reduce the risk of pharmacoresistant cases is an extremely important factor in the treatment of patients with SE. This report has educational, practical and organizational aspects, outlining a standard plan for SE management in Poland that will improve therapeutic efficacy.

Abstract: Publication date: Available online 1 June 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Jarosław Dulski, Michał Schinwelski, Agnieszka Konkel, Karol Grabowski, Witold Libionka, Piotr Wąż, Sitek E.J., Jarosław Sławek Aim of the studyWe present the preliminary results of the study focused on the impact of subthalamic deep brain stimulation (DBS-STN) on sleep and other non-motor symptoms (NMS).Materials and methodsTen patients with advanced PD, underwent two-night polysomnography (PSG) mean 1.1 week before surgery and 6.2 months post DBS programming. NMS were assessed with a set of scales before surgery and 6 months and 12 months following DBS programming.ResultsContrary to previous studies, we noted deterioration of sleep pattern in the follow-up PSG. We found a decrease in total sleep time, duration of the stage N2, with prolongation of stage N1 and wakefulness after sleep onset. We did not detect any impact of DBS-STN on subjective severity of restless legs syndrome. REM – sleep behavior disorder, however reported was not observed in any patient during PSG evaluations. We also found statistically significant correlations between severity of sleep disturbances and quality of life, as well as, between severity of motor symptoms and worse objective sleep quality.ConclusionsWe found that DBS-STN improved quality of life, subjective quality of sleep and sleepiness, however, contrary to the previous studies the objective parameters of sleep worsened after the surgery.

Abstract: Publication date: Available online 30 May 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Justyna Zielinska-Turek, Małgorzata Dorobek, Grzegorz Turek, Maria Barcikowska-Kotowicz We still lack an optimal tool to predict ischaemic stroke in patients with symptomatic and asymptomatic carotid stenosis (CS). It has already been shown that patients at increased risk of ischaemic stroke can be identified based on the elevated plasma levels of metalloproteinases (MMPs) and reduced activity tissue inhibitor of metalloproteinase (TIMP). There are few studies presenting the role of MMP-9 and TIMP in ischaemic stroke both in patients with symptomatic and asymptomatic CS treated with stenting or endarterectomy, however we have not found any published review summarizing the role of abovementioned markers. MEDLINE was accessed via Pub Med, and searched for published studies that analyzed MMP-9 and TIMP levels in patients with asymptomatic and symptomatic internal carotid stenosis and/or examined these parameters as potential risk markers for ischaemic stroke. A total of 13 articles documenting the outcomes of patients with symptomatic or asymptomatic carotid stenosis treated by carotid stenting or endarterectomy, were analyzed. Statistically significant differences in the levels of MMP-9 and/or TIMP in patients with symptomatic and asymptomatic CS have been reported. Also the concentrations of MMP-9 and TIMP in CS patients subjected to stenting or endarterectomy were higher than in baseline group. Moreover higher levels of MMP-9 and decreased TIMP was reported to be associated with the risk of restenosis. This systematic review shows that available evidence regarding the dynamics of MMP-9 and TIMP levels may be a predictor of cerebrovascular events in both symptomatic and asymptomatic carotid stenosis in patients treated with stenting or endarterectomy.

Abstract: Publication date: Available online 17 May 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Grzegorz Witkowski, Magdalena Konopko, Rafał Rola, Agnieszka Ługowska, Danuta Ryglewicz, Halina Sienkiewicz-Jarosz IntroductionLate-onset Pompe disease (LOPD) is a progressive metabolic myopathy, affecting skeletal muscles, which, if untreated, leads to disability and/or respiratory failure. The enzyme replacement therapy (ERT) improves muscle strength and respiratory function and prevents disease progression. We present a 6-year follow-up of 5 patients with LOPD treated with ERT.MethodsFive patients with LOPD received ERT: two started treatment in 2008, other two in 2010 and one in 2011. All patients received recombinant human alpha-glucosidase in dose 20 mg/kg intravenously every two weeks. Physical performance was assessed in 6-minute walk test (6MWT) and spirometry was performed to examine FVC and FEV1. Liver enzymes, CK levels were also assessed.ResultsThe walking distance in 6MWT increased by average 16.9 ± 2.26% in the first three years of treatment. Similar changes were detected in spirometry: the most significant FVC increase was observed in two patients with the highest FVC values before treatment, which increased to normal values adjusted for age and sex in three years of treatment, that is by 28% and 34%. In two other patients FVC reached 88% and 76% of predicted values. ERT also improved the liver and muscle enzymes levels.ConclusionThe improvements of exercise tolerance and FVC were observed in all patients in the first three years of treatment and were the most pronounced in the longest-treated patients and with the least severe neurological and respiratory symptoms. Our research suggests that early start of the ERT results in higher improvement of respiratory and ambulation functions.

Abstract: Publication date: Available online 14 May 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Agnieszka Wiertel-Krawczuk, Juliusz Huber PurposeTraumatic damage to the brachial plexus is associated with temporary or permanent motor and sensory dysfunction of the upper extremity. It may lead to the severe disability of the patient, often excluded from the daily life activity. The pathomechanism of brachial plexus injury usually results from damage detected in structures taking origin in the rupture, stretching or cervical roots avulsion from the spinal cord. Often the complexity of traumatic brachial plexus injury requires a multidisciplinary diagnostic process including clinical evaluation supplemented with clinical neurophysiology methods assessing the functional state of its structures. Their presentation is the primary goal of this paper.MethodsThe basis for the diagnosis of brachial plexus function is a clinical examination and neurophysiology studies: electroneurography (ENG), needle electromyography (EMG), somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) assessing the function of individual brachial plexus elements.ConclusionsThe ENG and EMG studies clarify the level of brachial plexus damage, its type and severity, mainly using the Seddon clinical classification. In contrast to F-wave studies, the use of the MEPs in the evaluation of traumatic brachial plexus injury provides valuable information about the function of its proximal part. MEPs study may be an additional diagnostic in confirming the location and extent of the lesion, considering the pathomechanism of the damage. Clinical neurophysiology studies are the basis for determining the appropriate therapeutic program, including choice of conservative or reconstructive surgery which results are verified in prospective studies.

Abstract: Publication date: Available online 8 May 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Abdulkadir Tunç, Aysel Kaya Tekeşin, Belma Doğan Güngen, Esra Arda IntroductionThis study aimed to evaluate the cognitive performance of migraine patients with (MwA) and without aura (MwoA) and investigate the correlation of white matter hyperintensities (WMHs) and psychological symptoms with their cognitive test scores.Material and methodsHundred migraine patients aged 20–55 years and 80 healthy volunteers with similar age, sex, and education level were enrolled. The total Montreal Cognitive Assessment (MoCA) scores were compared by age, sex, presence of aura, migraine duration, attack frequency, pain localization, presence and number of WMHs, and the scores of the Beck Depression Inventory and the Beck Anxiety Inventory (BAI).ResultsForty-seven (47%) patients had MwA and 53 (53%) had MwoA. The performance of the MwA patients was significantly poorer than that of the MwoA patients and the healthy subjects on the MoCA scales. In particular, the results revealed lower scores in the subscales regarding visuospatial/executive functions, naming, memory, attention, and abstraction in MwA patients than in the MwoA patients. Compared to healthy controls, more number of migraine patients had WMHs. The presence and number of WMHs had no significant correlation with the MoCA scores of the migraine patients. There was a significant correlation of the BAI and BDI scores with the total MoCA scores considering all migraine patients.ConclusionsThis study suggested that MwA may be associated with low cognitive performance which was correlated with depression and anxiety but not with WMHs. Further, longitudinal studies for assessing the relationship between WMHs, cognitive functions, and migraine, and for establishing the causality are warranted.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Simona Lattanzi, Mauro Silvestrini Posterior circulation stroke accounts for approximately 20% of all ischaemic strokes. Acute basilar artery occlusion (BAO) is one of the most severe conditions, it is associated with death or major disability in more than three quarters of the cases, and its optimal management remains unestablished. Currently, the treatment is based primarily upon consensus, the clinical practice varies widely, and the actual benefit of mechanical thrombectomy has to be fully estimated. Although the recent years have profoundly revolutionized and improved the stroke care, many questions still remain unanswered and will represent the challenges of the next future.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Anetta Lasek-Bal, Joanna Kidoń, Monika Błaszczyszyn, Bartłomiej Stasiów, Amadeusz Żak Functional magnetic resonance imaging (fMRI) allows for the assessment of neuronal activity through the blood-level-dependent signal. The purpose of study was to evaluate the pattern of brain activity in fMRI in patients with ischemic stroke and to assess the potential relationship between the activity pattern and the neurological/functional status.MethodsThe fMRI was performed in patients up to 4th day of stroke. All the patients were analyzed according to NIHSS on 1st day and mRankin scale on 14th day of stroke, followed by analyzing of fMRI signal.ResultsThe study enrolled 13 patients at a mean age of 64.3 years. Eight (61.5%) showed cerebellar activation and 2 (15.38%)- insular activation. In those who scored 0–2 on mRankin scale, the most frequently observed activity was located in the regions: the M1, SMA and PMC in the stroke hemisphere and the cerebellum. In those cases, the non-stroke hemisphere was more frequently involved in the areas: the M1 and PMC. There was a tendency for a better prognosis in relation to age

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Andżelina Wolan-Nieroda, Andrzej Maciejczak, Grzegorz Przysada, Adrian Kużdżał, Grzegorz Magoń, Michlina Czarnota, Mariusz Drużbicki, Agnieszka Guzik BackgroundSurgical treatment of odontoid fractures with posterior C1/C2 fusion always leads to severe limitations in mobility of the cervical spine and head.PurposeTo assess the mobility of the cervical spine in patients treated with various surgical methods after an axis body fracture.Material and methodsA group of 61 subjects receiving surgical treatment in a group of 214 subjects treated for odontoid fractures at one ward of neurosurgery at a regional hospital. Studies also included odontoid peg and Hangman fractures. The range of motion of the head was compared to standards by the International Standard Orthopedic Measurements (ISOM) and to head mobility in a control group of 80 healthy subjects without any pathologies or complaints associated with the cervical spine. Ranges of motion were measured with the CROM goniometre with regard to flexion, extension, right and left lateral flexion and right and left rotation. The functional status was evaluated with Neck Disability Index (NDI) standard questionnaires indicated for patients with cervical spine pain.ResultsExcept for flexion and extension, patients after odontoid fractures had a statistically significantly smaller range of motion of the cervical spine in all planes compared to the control group and ISOM standards.ConclusionsOdontoid fractures lead to limitations in mobility of the cervical spine even after treatment with methods that in theory should preserve the C1/C2 mobility.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Natalia Chmielewska, Janusz Szyndler, Karolina Makowska, Dawid Wojtyna, Piotr Maciejak, Adam Płaźnik The role of blood brain barrier (BBB) is to preserve a precisely regulated environment for proper neuronal signaling. In many of the central nervous system (CNS) pathologies, the function of BBB is altered. Thus, there is a necessity to evaluate a fast, noninvasive and reliable method for monitoring of BBB condition. It seems that revealing the peripheral diagnostic biomarker whose release pattern (concentration, dynamics) will be correlated with clinical symptoms of neurological disorders offers significant hope. It could help with faster diagnosis and efficient treatment monitoring. In this review we summarize the recent data concerning exploration of potential new serum biomarkers appearing in the peripheral circulation following BBB disintegration, with an emphasis on epilepsy, traumatic brain injury (TBI) and stroke. We consider the application of well-known proteins (S100β and GFAP) as serum indicators in the light of recently obtained results. Furthermore, the utility of molecules like MMP-9, UCHL-1, neurofilaments, BDNF, and miRNA, which are newly recognized as a potential serum biomarkers, will also be discussed.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Żanna Pastuszak, Zbigniew Czernicki, Waldemar Koszewski, Adam Stępień, Anna Piusińska-Macoch IntroductionMalignant ischemic stroke of the middle cerebral artery (MCA) territory causes neurological deterioration due to the effects of space occupying cerebral edema. The prognosis is poor, and death usually occurs as a result of brainstem compression. There is no information on ischemic stroke, especially the malignant ones, in patients over 85 years old.AimThe aim of this retrospective study was to evaluate the disease course, risk factors, survival rate and treatment of MCA malignant infarction in people over 85 years old.MethodThe medical history of 66 patients with malignant MCA stroke was analyzed. The frequency of the occurrence of the risk factors like hypertension, hyperlipidemia, atrial fibrillation, heart failure, diabetes was evaluated. Disability was measured with the use of the National Institutes of Health Stroke Scale (NIHSS). Safety and effectiveness of the anticoagulants used in the group of patients with atrial fibrillation were analyzed. Chi-quadrat test and Mann–Whitney U test were used for statistical analysis of data. We also described 85 year-old patient with malignant brain stroke who was treated neurosurgically with a positive effect.ResultsAtrial fibrillation was diagnosed in 65% of patients of the investigated group. There were no statistically significant changes in the survival rate between the group of patients treated with the use of mannitol and patients without this treatment.ConclusionThe key risk factor in this group is the atrial fibrillation. The elderly patients require an intensive monitoring of the health condition by reference to brain stroke risk factors, especially atrial fibrillation.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Dariusz Szczepanek, Ewa Wąsik-Szczepanek, Agnieszka Szymczyk, Tomasz Gromek, Ewelina Grywalska, Monika Podhorecka, Marek Hus Secondary nervous system lymphoma (SCNSL) is a rare extranodal form of non-Hodgkin lymphoma (NHL). This applies to a particular form of lymphoma that does not originally derive from the central nervous system (CNS); it can be both an isolated form of relapse or a systemic part of disease progression. Due to poor prognosis and a lack of established algorithms of therapeutic procedures, it is a big challenge for physicians from many specializations. In our study, we present an interesting case of a patient with a relapsed form of SCNSL for whom a unique form of treatment was used – intraventricular administration of rituximab and methotrexate.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Simona D. Marino, Laura Schiavone, Flavia M.C. La Mendola, Tiziana Timpanaro, Maria Elena Cucuzza, Filippo Greco, Pierluigi Smilari, Agata Fiumara, Andrea Domenico Praticò Unilateral palsy of the hypoglossal nerve is a rare complication of orthodontic procedures. The main reported causes of HNP are: orthopedic and otorhinolaryngology surgical interventions, and in particular maneuvers involving compression or overstretching of the hypoglossal nerve, dental procedures and traumas, and also infections, motoneuron disorders, tumors, vascular diseases. Diagnosis is usually performed by electrophysiology studies (EMG-VCN), and brain magnetic resonance imaging (MRI) is useful to exclude other causes. The prognosis depends on the location and extension of the damage. Currently there is not a standardized treatment approach except the speech therapy, although, in some cases, the high-dose steroid treatment could be useful. We describe the case of a ten-year-old female, who was admitted in our Unit after a deviation of the tongue associated with dysarthria and dysphagia, occurred after the application of a mobile orthodontic device.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Claudia Stöllberger, Nicolas DeCillia, Josef Finsterer BackgroundEmbolic stroke is a complication of Tako-Tsubo-cardiomyopathy (TTC), transient left ventricular dysfunction mimicking myocardial infarction without coronary culprit lesion explaining the whole left ventricular dysfunction. Transient global amnesia (TGA) is characterized by sudden onset of anterograde amnesia without compromise of other neurologic functions.Case reportA 57 years old female was admitted because of sudden confusion and loss of her memory after emotional stress. TGA was diagnosed. Because of chest pain and laboratory tests indicating myocardial ischemia, she underwent coronary angiography which was normal. Within the following 24 h her memory improved. She complained about severe vertigo and cerebral magnetic resonance imaging showed a recent ischemia in the left cerebellum.ConclusionsTGA is not a disease for the neurologists exclusively but also cardiac comorbidities have to be considered and cardiologists should be involved in the acute care of these patients in order not to overlook life-threatening diseases.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Shaolin Zhang, Jinsheng Huang, Yanling Chen BackgroundIntracranial involvement is an uncommon manifestation of Rosai–Dorfman disease (RDD) and had been rarely reported. In this study, we explore clinical characteristics, imageology manifestations and pathological features of primary intracranial RDD so as to improve the understanding for this disease.MethodsOne case (16-years-old boy) with primary intracranial RDD was analyzed and studied retrospectively by MRI features, histopathological observation and immunohistochemical staining, and the related literatures were reviewed.ResultsThe case was single lesion and involved the dura of the left middle cranial fossa base, which was iso-hypo signal intensity on T1WI and hypointense on T2WI and FLAIR image. The lesion was a homogeneous contrast enhancement mass with dural tail sign and had peritumoral brain edema. Pathological analysis showed the lesion consisted of variable numbers of mature lymphocytes, plasma cells and neutrophils. The characteristic histiocytes were emperipolesis and positively expressed for S-100 and CD-68 and negatively expressed for CD-1a by immunohistochemical analysis. Based on clinical presentations and histological findings after surgical excision, a final diagnosis of primary intracranial RDD was made.ConclusionPrimary intracranial RDD, especially located in the cranial base, is exceptionally rare, which hard to be distinguished with meningoma by imageology and clinical manifestations, but could be diagnosed by pathological and immunohistochemical examinations. Surgery is of the most importance treatment and prognosis is optimistic for this disease.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Takuya Konno, Patrick R. Blackburn, Todd D. Rozen, Jay A. van Gerpen, Owen A. Ross, Paldeep S. Atwal, Zbigniew K. Wszolek Aim of the studyTo describe a family with primary familial brain calcification (PFBC) due to SLC20A2 variant showing possible genetic anticipation.Materials and methodsWe conducted historical, genealogical, clinical, and radiologic studies of a family with PFBC. Clinical evaluations including neurological examination and head computed tomography (CT) scans of a proband and her father were performed. They provided additional information regarding other family members. To identify a causative gene variant, we performed whole-exome sequencing for the proband followed by segregation analysis in other affected members using direct sequencing.ResultsIn this family, nine affected members were identified over four generations. The proband suffered from chronic daily headache including thunderclap headache. We identified an SLC20A2 (c.509delT, p.(Leu170*)) variant in three affected members over three generations. Interestingly, the age of onset became younger as the disease passed through successive generations, suggestive of genetic anticipation.Conclusions and clinical implicationsFor clinical purpose, it is important to consider thunderclap headache and genetic anticipation in PFBC caused by SLC20A2 variants. Further investigation is required to validate our observation.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Marlena Hupalo, Rafal Wojcik, Dariusz J. Jaskolski BackgroundResults of DBS of ATN in refractory epilepsy depend on accuracy of the electrode's location. We searched for characteristic intraoperative, intracerebral EEG recording pattern from anterior thalamic nuclei (ATNs) as a biological marker for verifying the electrode's position.MethodsThere were six patients with refractory epilepsy scheduled for deep brain stimulation (DBS) procedure. At surgery, to map the target, we recorded EEG from each lead of DBS electrodes. One patient underwent a 24 hours EEG with continuous recording from both ATNs before internalization of stimulator units.ResultsIn all patients we recorded spontaneous bioelectric activity of ATNs. The pattern of the recording from the ATN was similar in all cases. In the one patient where 24-hour recording was done with simultaneous scalp EEG, a complex partial seizure was captured.ConclusionThis is the first report of using DBS electrode for intraoperative EEG recordings from the ATN in patients with refractory epilepsy. Since we managed to find the characteristic pattern of bioelectric activity of ATN, this technique seems to be a promising method for targeting this structure during the operation.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Izabela Monika Rozmilowska, Monika Helena Adamczyk-Sowa, Damian Czyzewski IntroductionThe Myasthenia Gravis-Activities of Daily Living scale (MG-ADL) is a short, and easy to use disease-specific quality of life during daily routine tool in myasthenia gravis.ObjectivesThe purpose of our work was to evaluate neurological condition patients with myasthenia gravis using the form MG-ADL in order to enable the introduction in common use of an instrument which allows for the assessment of patients with myasthenia gravis.Patients and MethodsThe total number of 50 patients with MG were qualified for the examination. Each patient underwent neurological examination and completed the quality of life evaluation questionnaire MQ-ADL. Additionally, each patient was asked to evaluate the quality of his/her life by means of questionnaire MG-QOL 15 and MG Composite in Polish language version.ResultsOur analysis showed a positive correlation with other scales used - MG-QOL 15, MGFA, MG Composite. The intensification of neurological symptoms showed significant relation with obtained higher number of points in MG-ADL questionnaire. The MG-ADL was found to have high internal consistency, test–retest reliability, and concurrent validity.ConclusionWe confirmed reliability and dependability of the questionnaire in the the test-retest assessment. The MG-ADL is accepted to be a reliable and valuable tool for measuring disease-specific QOL in Polish patients with MG.

Abstract: Publication date: May–June 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 3Author(s): Burcu Polat, Ozlem Saatci, Nesrin Helvaci Yilmaz, Ozge Arici Duz BackgroundIt is known that complementary and integrative medicine (CIM) methods are especially used by patients with chronic headaches. The aim of our study is to increase the knowledge on this topic, to provide objective data about use in Turkish headache patients.MethodsThis study included 425 patients with headache. The survey form prepared was filled in under the supervision of a health professional. The questionnaire included 2 items, about CIM methods and finance.ResultsAmong the patients evaluated, 316 were female, and 109 were male. All of 52% answered yes to the question “did you ever use any CIM treatment method for headaches during your life'”. The most frequently used methods were combined (herbal + one or more other method) (29.6%), herbal (9.4%) and cupping therapy (4.2%). Among the patients that used combined methods, 26.9% had spent 30–100 TL (5–25 euro), 20.6% had spent 100–300 TL (25–70 euro), 26.9% had spent 300–500 TL (70–120 euro) and the last two groups that formed 12.6% had spent 500–1000 (120–250 euro) and>1000 TL (>250 euro).ConclusionHalf of the patients that applied to outpatient clinic with headaches use one or more of these methods and make budgets in accordance with their income levels. Physicians should have sufficient knowledge and clinical opinions about the CIM methods used by headache patients.

Abstract: Publication date: Available online 29 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Alicja Kalinowska-Łyszczarz, Mikołaj A. Pawlak, Aleksandra Wyciszkiewicz, Krystyna Osztynowicz, Wojciech Kozubski, Sławomir Michalak Although neurons are the main source of neurotrophins in the healthy brain, neurotrophins can also be expressed in the immune system. We have previously shown that in relapsing-remitting multiple sclerosis (RRMS) lower immune-cell neurotrophin levels are associated with brain atrophy and cognitive impairment. The aim of the present study was to assess if immune-cell neurotrophin expression is impaired in MS as compared with the healthy controls, and to describe if these levels change in treatment-naïve RRMS patients, following one year of immunomodulation.Fifty treatment-naïve RRMS patients were assessed at baseline and after one year of immunomodulation (beta-interferons/glatiramer acetate). The control group included 39 healthy subjects matched according to age and gender. Peripheral blood mononuclear cells (PBMCs) were isolated from heparinized blood using Ficoll-Histopaque gradient. The levels of brain-derived-neurotrophic-factor (BDNF), beta-nerve-growth-factor (beta-NGF), neurotrophin-3 (NT-3) and neurotrophin-4/5 (NT-4/5) were measured in PBMC lysates with ELISA.BDNF levels were significantly lower in MS than in the healthy controls (median 613 vs. 1657 pg/mg protein, p

Abstract: Publication date: Available online 17 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Leszek Lombarski, Przemysław Kunert, Marcin Skawiński, Marek Prokopienko, Zbigniew Lewandowski, Andrzej Marchel ObjectiveVertebral artery medulla compression syndrome (VAMCS) is a very rare condition manifesting as different neurological focal deficits. The case of a 36-year-old male with symptomatic brainstem compression by vertebral artery (VA) treated by means of microvascular decompression (MVD) and a review of the literature is presented.Case reportOn admission, a 36-year-old patient presented with hypoalgesia, hypothermesthesia and hemiparesis on the left side. Magnetic resonance imaging (MRI) of the head disclosed the right VA loop compressing the ventrolateral medulla and excluded other entities such as brain tumor, stroke and multiple sclerosis. Since displacement and significant compression of the right pyramidal tract was confirmed by diffusion tensor imaging (DTI), neurovascular compression syndrome was diagnosed. The patient underwent MVD of the medulla using a Gore-Tex implant as a separating material via the right far-lateral approach. The left hemiparesis and hemisensory loss remitted rapidly after the procedure. The post-procedural neurological improvement was maintained at one year follow-up. Based on a review of the literature, a total of 33 cases of surgically treated VAMCS has been reported so far.ConclusionVAMCS should be considered as the cause of neurological deficits when other pathological entities are ruled out. In symptomatic conflict of the VA with the medulla, microvascular decompression using a Gore-Tex implant can be an effective method of treatment. Nevertheless, a statistical analysis on all reported cases showed favorable results using the VA repositioning technique when compared with MVD (success rate 91% vs. 58%, p

Abstract: Publication date: Available online 15 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Nexhmedin Shala, Shemsedin Dreshaj IntroductionMigraine is ranked as the seventh leading cause of disability worldwide, and it is characterized by a manifestation of combined neurological, gastrointestinal, and autonomic symptoms linked with different provoking factors.Aim of the studyThis study investigates the association between migraine and PTSD, depression and anxiety in the Kosovo population during the post-war period.Material and methods273 war survivors with headache were enrolled in the study and were divided into two groups: 153 individuals with confirmed migraine (the study group) and 120 individuals with non-migraine headaches (control group). All individuals were evaluated using the ICHD-II 2004 diagnostic criteria for migraine, as well as the mini-test for PTSD, MMPI (Minnesota Multiphasic Personality Inventory) for psychological evaluation, PAI (Personality Assessment Inventory) and Hamilton Scale for Depression.ResultsAmong migraine patients, depressive disorders were present in 27.5% of patients, anxiety was found in 60.8%, and PTSD was present in 39.2%. While the prominence of depression was not different between groups, anxiety was significantly more common (p

Abstract: Publication date: Available online 13 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Cahit Kural, Gokce Kaan Atac, Ozkan Tehli, Ilker Solmaz, Caglar Temiz, Irgen Hodaj, Yusuf Izci ObjectiveTo investigate the effects of dexamethasone on brain tumor and peritumoral edema by different sequences of magnetic resonance imaging (MRI).Materials and methodsMRI was performed in 28 patients with brain tumor. Patients were divided into the 3 groups based on the histological diagnosis; Group I: high-grade glial tumor, Group II: low-grade glial tumor, and Group III: brain metastasis. The measurements of peritumoral edema volume and apparent diffusion coefficient (ADC) values were performed while the peak areas of cerebral metabolites were measured by spectroscopy in groups I and II. The changes in edema volumes, ADC values and cholin/creatine peak areas were compared.ResultsThe volume of peritumoral edema was decreased in groups I and II, but increased in group III after dexamethasone treatment. These changes were not statistically significant for 3 groups. ADC value was decreased in group I and increased in groups II and III. Changes in ADC values were statistically significant. Cholin/creatine peak areas were decreased after dexamethasone in groups I and II, but these changes were also not significant.ConclusionDexamethasone has no significant effect on the volume of peritumoral edema in glial tumor and metastasis. Moreover, dexamethasone increases the fluid movements in low grade gliomas and metastases, decreases in high grade gliomas. However, more comprehensive clinical studies are needed to show the effects of dexamethasone on brain tumors and peritumoral edema.

Abstract: Publication date: Available online 9 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Seidu A. Richard, Lu Ma, Hao Li, Jin Li, Chao You IntroductionSpinal arteriovenous malformations (SAVMs) are very rare and can be very challenging to treat since none of the therapeutic options does provide a definitive cure to these lesions. We believe that incorporation of intraoperative angiography during surgery in a hybrid theatre can help achieve a better cure.Case presentationWe present a 45 years old woman with three (3) years history of weakness and ten (10) days’ history of acute pain in right upper extremity. Magnetic resonance angiography (MRA) of the cervical segment of spinal cord revealed tortuous vascular masses from foramen magnum to the inferior margin of fourth cervical (C4) vertebral. Spinal digital subtracting angiography (DSA) confirmed vascular malformation at the cervical segment of the spinal cord with their origin from bilateral posterior spinal arteries. She was successfully operated on with the aid of intraoperative angiography without any neurological deficient.ConclusionSpinal angiography is the gold standard for all-inclusive assessment of SAVMs. Surgery and endovascular techniques equally have key therapeutic valves in treatment of SAVMs but a combination of the two gives a more accurate and reliable cure to this disorder.

Abstract: Publication date: Available online 8 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Agnieszka Niemierzycka, Krzysztof Tomczuk, Mirosław Mikicin, Agnieszka Zdrodowska, Grzegorz Orzechowski, Marek Kowalczyk ObjectiveThe article represents the preliminary study, with the aim of the experiment being to examine whether different types of light sources used commonly in building interiors combined with various color temperature have an effect on EEG activity. The effect of frequency pulsation and color temperature on brain activity in EEG examinations in the beta 2 band was assumed.Material/participantsTwenty healthy men aged 19–25 years participated in the experiment.MethodsThe research stand was lit by: LED diodes with color temperatures of 3000 K, 4200 K, 6500 K, with the power supplied using the pulse width modulation (PWM) method with the current frequency of 122 Hz, linear fluorescent tubes (3000 K, 6500 K), with the power supplied with the frequency of 50 Hz and 52 kHz from the electromagnetic and electronic ballasts, and the conventional light bulb, with the power supplied directly from the mains electricity, used as a reference light. System Flex 30 apparatus with TrueScan software was used to record the EEG signal. The examination used two factors (speed and accuracy) of the Kraepelin's work curve to describe changes in work performance for various types of lighting.ResultsThe results demonstrate that the use of different types of emission of light and color temperature of the light have an effect on bioelectrical brain activity and work performance.ConclusionsThe highest activity of brain waves concerns the beta band in the frequency range of 21–22 Hz, regardless of the type of the light source (LED, fluorescent tube). The methods used to supply power and color temperature of fluorescent tubes do not significantly affect bioelectrical brain activity during “work”, but previous lighting with fluorescent tubes during work has an essential effect on bioelectrical brain activity during rest. Regardless of the color temperature, LED lighting with PWM power supply leads to the highest bioelectrical activity (mainly in the range of 21–22 Hz) in the brain during work and rest, which might suggests the usefulness of this method of supplying power for everyday work. Incandescent light does not affect the bioelectrical brain activity during work and rest.

Abstract: Publication date: Available online 3 March 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Kyriakos Paraskeva, Dimitrios Giakoumettis, Ioannis Nikas, George Georgoulis, George Sfakianos, Marios S. Themistocleous IntroductionNeck pain and torticollis are common symptoms in the pediatric population that rarely requires further investigation. However, in case symptoms persist, then a more meticulously approach should be considered. Underlying conditions such as infections, neck injury, autoimmune disorders or even cervical spine cancer should be excluded from diagnosis. Cervical spine cancer is a rare neurosurgical entity in the pediatric population and even rarer is atlantoaxial Ewing's sarcoma. In this report, we present a rare case of primary Ewing's sarcoma of the axis.Case reportA 3.5-year-old female with progressive neck pain and intermittent episodes of torticollis was referred to our outpatient clinic. Imaging studies revealed a malignant tumor located on C2 vertebra. Diagnosis of Ewing's Sarcoma was confirmed via open biopsy and the patient was treated with Euro-EWING 99 chemotherapy.ConclusionPediatric neck pain and/or torticollis should raise high suspicion for malignancy of cervical spine. Modern diagnostic means and techniques can assist in the screening and diagnosis of these tumors.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Marta Skowronska, Anna Piorkowska, Anna Czlonkowska ObjectivesThere is little data about sex differences in carotid atherosclerosis in the early phase after an ischemic event. The aim of this study was to examine the carotid artery atherosclerosis differences between men and women in early phase after TIA or stroke.MethodsConsecutive patients with recent ischemic event, admitted during the first week after symptom onset were examined with ultrasound. Sex differences in degree of stenosis, number of plaques and plaque morphology were compared. Plaque morphology was assessed by gray-scale median (GSM), according to which lower values were associated with hemorrhagic/necrotic core indicating plaque instability.ResultsOf the 316 patients with ischemic events, 196 (50.5% male) entered the study. Men had more often moderate as well as severe ipsilateral carotid stenosis (12.1% vs 7.2% for moderate and 12.1% vs 2.1% for severe; p = 0.024). Men had more often the largest plaque hypoechogenic contralateral (62.6% vs 37.1%, p = 0.0008), but not ipsilateral. Men had 3 or more hypoechogenic plaques (24.2% vs 4.1%, p = 0.0001; 17.2% vs 4.1%, p

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Anetta Lasek-Bal, Teresa Kowalewska-Twardela, Aldona Warsz-Wianecka, Przemysław Puz, Aleksandra Krzan, Aleksandra Cieślik BackgroundThe efficacy and safety of thrombolytic therapy in stroke depend on multiple factors. The aim of this study was to evaluate the significance of atrial fibrillation the prognosis in terms of the functional status in patients with stroke treated with intravenous thrombolysis. An additional aim was also to assess the potential significance of reduced ejection fraction (EF) and enlarged left atrium (LA) of the heart for the prognosis in patients with stroke who underwent thrombolytic therapy.MethodologyA prospective study involved enrollment of 222 patients, mean age of 72 years with first-in-life ischemic stroke. In all participants there were realized procedures as follows: neurological status before administering rt-PA (NIHSS), selected echocardiographic parameters, functional status on the 14th day from the onset (mRankin scale) and analysis the bleeding events.ResultsAtrial fibrillation was significantly more frequent in women than men; females had higher CHA2DS2VASc scores and heavier neurological conditions on day 1 of stroke. Two independent factors for poor prognosis (3–5 points by mRankin) were found: the NIHSS score and the CHA2DS2VASc score ≥3. We identified 2 independent factors for death within 14 days from the onset: the result by NIHSS and the EF. The NIHSS score turned out to be the only independent predictor of hemorrhage during hospitalization: RR 1.19; CI [1.06–1.33]; p = 0.003; p for model = 0.0025.ConclusionsThe presence of atrial fibrillation worsens the patient's prognosis in terms of the functional status and survival during the acute period of stroke in patients treated with intravenous thrombolysis.Higher NIHSS and CHA2DS2VASc scores and reduced EF in patients with stroke treated with thrombolysis are the predictors of unfavorable short-term prognosis.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Geneieve Tai, Louise A. Corben, Eppie M. Yiu, Sarah C. Milne, Martin B. Delatycki Friedreich ataxia (FRDA) is a progressive neurological disorder affecting approximately 1 in 29,000 individuals of European descent. At present, there is no approved pharmacological treatment for this condition however research into treatment of FRDA has advanced considerably over the last two decades since the genetic cause was identified. Current proposed treatment strategies include decreasing oxidative stress, increasing cellular frataxin, improving mitochondrial function as well as modulating frataxin controlled metabolic pathways. Genetic and cell based therapies also hold great promise. Finally, physical therapies are being explored as a means of maximising function in those affected by FRDA.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Cecylia Rajczewska-Oleszkiewicz, Agnieszka Cyganek, Anna Stadnik, Dorota Dziewulska We describe an 86-year-old woman with a history of hypertension who presented sudden disturbances of consciousness and left hemiparesis. Brain magnetic resonance imaging (MRI) revealed diffused hyperintensive changes on T2-weighted images localized subcortically in the white matter of both cerebral hemispheres, corresponding to acute vasogenic edema, causing moderate mass effect. Posterior reversible encephalopathy syndrome was initially diagnosed. After implementation of anti-edema intravenous steroid treatment and hypotensive therapy the symptoms began to retire, till the total regression. The successive hospitalizations took place two and eight months later due to the occurrence of seizures, motor deficits and the development of mild cognitive impairment. Brain MRI revealed progression of the white matter changes and diffused subcortical microhemorrhages. Each time pulse steroid therapy was implemented and the symptoms improved significantly after several days. Chronic oral steroid treatment resulted in the stabilization of neurological status. The long-term observation of clinical symptoms, remission after immunosuppressive therapy and white matter changes with subcortical microhemorrhages in brain MRI leaded to the diagnosis of cerebral amyloid angiopathy-related inflammation.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Joyutpal Das, James B. Lilleker, Kavaldeep Jabbal, John Ealing Spinal muscular atrophy (SMA) is a hereditary neuromuscular disorder, which causes progressive muscle weakness and in severe cases respiratory failure and death. Although the majority of the SMA cases are autosomal recessive, there is an autosomal dominant variant of SMA that primarily affects the lower extremities, known as ‘spinal muscular atrophy – lower extremity, dominant’ (SMALED). Mutations in the Dynein Cytoplasmic 1 Heavy Chain 1 (DYNC1H1) gene were the first to be associated with SMALED. Here we report a family with SMALED caused by a pathogenic heterozygous missense c.1809 A > T, p.glu603Asp mutation in DYNC1H1. The main clinical features were congenital hip displacement, talipes, delayed motor development, wasting and weakness in lower limbs with relative sparing of upper extremities and very slow disease progression.SMALED is extremely rare and only a handful of families have been reported. Over the years other phenotypes including Charcot Marie Tooth type 2 and hereditary mental retardation with cortical neural migration defects have also been reported to be caused by DYNC1H1 mutations.This report aims to increase our awareness of SMALED and various other phenotypes associated with mutations in this gene.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Mehmet Osman Akçakaya, Nihan Hande Akçakaya, Mustafa Ömür Kasımcan, Talat Kırış Parkinsonism-hyperpyrexia syndrome (PHS), or neuroleptic malignant syndrome (NMS), is a neurophysiologic reaction to the acute withdrawal/decrease of central dopamine levels. It is a severe complication characterized by rigidity, change in consciousness level, fever, hypertension, and autonomic instability, that can be fatal. To the best of our knowledge, PHS following deep brain stimulation (DBS) of subthalamic nucleus (STN) surgery due to anti-Parkinson drug discontinuation has been previously reported only six times. Half of these cases resulted in fatalities. Herein, we report on an early diagnosed case of PHS following bilateral STN-DBS which was successfully treated with the administration of dopamine agonists, fluid replacement, and activation of DBS.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Zehra Aktan, Nihan Hande Akcakaya, Pinar Tekturk, Engin Deniz, Bahar Koyuncu, Zuhal Yapici Charcot-Marie-Tooth (CMT) disease is a hereditary neurologic disease which affects the sensorial and motor fibers of the peripheral nerves. CMTX1 is an X-linked dominantly inherited subtype of CMT and is caused by mutations in gap junction beta 1 gene (GJB1). A small proportion of GJB1 mutations are associated with recurrent central nervous system findings. We describe a 15-year-old male patient with CMTX1 who had stroke-like findings along with foot deformities and peripheral neuropathy. Strokes and stroke-like attacks are rarely seen in children and adolescents. Herein, neurological signs, MRI findings and genetic results of a CMTX1 case are presented and discussed.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Żanna Pastuszak, Renata Piusińska-Macoch, Adam Stępień, Zbigniew Czernicki BackgroundPost polio syndrome is a rare disease that occurs decades after polio virus infection. Repetitive transcranial magnetic stimulation (rTMS) is a treatment option with proved effectiveness in drug resistant depression. Possibly it can be helpful in therapy of other neurological diseases including post polio syndrome.ObjectiveTo describe a case of patient diagnosed with post polio syndrome who was treated with rTMS stimulation with a good effect.MethodsPatient had rTMS stimulation of left prefrontal cortex twice a week for an eight weeks. Patient's health status was evaluated before treatment, after last rTMS session and after three months from the end of the treatment.ResultsImprovement of fatigue score, mood disturbances and motor functions was observed after treatment.ConclusionrTMS can be an effective method in treatment of post polio syndrome but further studies with larger group need to be done to confirm that data.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Saeed Alghamdi, Yehya Seddeq, Hussein Algahtani, Bader Shirah Marchiafava–Bignami disease (MBD) is a rare fatal neurological disorder characterized by demyelination, primary degeneration, and necrosis of the corpus callosum. Although MBD is mostly associated with chronic alcohol consumption and malnutrition, it has been reported in non-alcoholic patients. Serotonin syndrome is a rare but potentially fatal side effect of antidepressants that results from overstimulation of both central and peripheral serotonergic receptors. In this report, we present a case with fatal serotonin syndrome happening in a non-alcoholic patient with the chronic form of MBD. To our knowledge, this case is the first report of fatal serotonin syndrome due to citalopram in an MBD patient. The present report may indicate that citalopram and other SSRIs should not be used in patients with MBD. Our case is also among few reported cases in the literature where no cause was identified in a patient with no previous history of alcohol intake.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Mehmet Seçer, Murat Ulutaş, Fatih Alagöz, Kadir Çınar, Özhan Merzuk Uçkun, Ergün Dağlıoğlu Cavernous Hemangiomas (CH) arise from any part of the cerebrum. Intraventricular lesions are rare and pure third ventricular localization is rare for CHs. Cavernomas of the ventricles may present with bleeding or signs associated with hydrocephalus. Radical excision is advocated for optimal management of cavernomas.In the present study, we present two cavernomas of the third ventricle which were completely excised via interhemispheric transcallosal transforaminal and Sylvian approaches. Radical excision should be the aim of the surgeon since reoperation for residual cavernomas is more commonly associated with complications and poor outcome.Total excision should be the goal of the treatment due to risk of rebleeding or regrowth.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Magdalena Torbus-Paluszczak, Sebastian Mucha, Maciej Wawrzyńczyk, Krystyna Pierzchała, Wojciech Bartman, Monika Adamczyk-Sowa Internal carotid artery dissection (ICAD) has become an increasingly recognized cause of cerebrovascular accidents in young and middle-aged patients. We report 2 cases of hypoglossal nerve palsy in the course of dissection of the internal carotid arteries. The first patient was admitted to the Department of Neurology due to swallowing difficulty, speech articulation disorders and numbness of the right half of the tongue for 4 weeks. Extracranial vessel ultrasound (US) and transcranial colour Doppler (TCD) visualized thrombus causing occlusion of the right internal carotid artery (RICA). Angio-CT revealed a compression on right XII nerve and a dissection of the RICA. The second patient was referred to the Department of Neurology due to articulation disorders and swallowing difficulties. On admission, neurological examination revealed tongue deviation towards the right side with evidence of atrophy of the right half of the tongue, deviation of the uvula to the right side, absence of palatal and pharyngeal reflexes, rhinolalia and dysphagia. Vessel imaging was taken using angio-MR showing mural thrombus of the RICA.ConclusionThe diagnosis of spontaneous non-traumatic dissection of the carotid arteries is a major challenge for clinicians. ICAD must be considered for young and middle-aged patients when severe headache is preceded by the co-existence of focal neurological symptoms. The probability of ICAD increases in the presence of predisposing diseases. The final diagnosis is based on imaging studies: color duplex ultrasound, CT angiography or MR angiography.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Michalina Malec-Litwinowicz, Andrzej Plewka, Danuta Plewka, Edyta Bogunia, Michał Morek, Andrzej Szczudlik, Michał Szubiga, Monika Rudzińska-Bar IntroductionParkinson disease (PD) is the common neurodegenerative disease. α-Synuclein (ASN), main aggregating protein in neural cells of CNS in PD, was found in peripheral fluids. Testing ASN in plasma is potential test for diagnose PD, but previous studies are controversial. The aim of this study was to investigate if plasma ASN level may be a valuable biomarker, is the level of plasma ASN concentration different in various motor subtypes of diseases, is there a relation between the level of plasma ASN and the severity of motor symptoms.MethodsPatients with PD hospitalized in Neurology Department, Medical College were performed sequencing the 8th and 9th exon of GBA gene. Next plasma ASN level was tested in 58 patients with sequenced GBA gene and in 38 healthy volunteers (HV), matched by the age (respectively 68.43 vs. 64.57 years of age) and sex (female %, respectively: 43.10 vs.44.74). Patients were assessed with the scales: UPDRS (II, III, IV), Hoehn–Yahr (HY) and qualified to PIGD or TD subtype. For homogeneity of the group patients with GBA mutation were excluded from the analysis.ResultsThe ASN level did not differ between patients and HV (respectively: 4.53 vs. 3.73 ng/ml) and between patients with different subtypes. There was inverse correlation between ASN and HY in PIGD subtype.ConclusionsPlasma ASN level is not valuable marker of the disease. It does not differ in subtypes of the disease. There is relation between plasma ASN level and the severity of the disease in PIGD subtype.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Dariusz Szczepanek, Ewa Wąsik-Szczepanek, Agnieszka Szymczyk, Małgorzata Wach, Maria Cioch, Monika Podhorecka, Ewelina Grywalska, Marek Hus Inclusion of the central nervous system (CNS) in the course of chronic lymphocytic leukaemia (CLL) is rare. At the moment no risk factors or proven treatment methods are known. The disease is described both in its early phase and during its acceleration period, thus it has been suggested that there might be independent mechanisms influencing the development of this condition. As there are no unified diagnostic procedure algorithms each patient needs to be assessed individually. CLL can manifest mostly in elderly people, for whom a possibility of development of neurological disorders with their aetiology different from leukaemia, should also be taken into consideration. The thesis presents a group of seven patients with CLL with CNS infiltration. Patients with prolymphocytic leukaemia, Richter's transformation and the original location of leukemic infiltration within the eye socket constitute an especially interesting case.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Katarzyna Bienias, Joanna Ścibek, Joanna Cegielska, Jan Kochanowski Slowly progressive neuromuscular diseases include but are not limited to: facioscapulohumeral muscular dystrophy (FSHD) and limb-girdle muscular dystrophy (LGMD), hereditary motor and sensory neuropathy (HMSN) and spinal muscular atrophy type III (SMA3). The purpose of this study is to present an evaluation of basic and complex activities of daily living in patients suffering from these diseases.The study was conducted on a group of 58 Polish patients: 25 patients with HMSN, 19 with LGMD and FSHD and 14 with SMA3. The research instrument consisted of two parts: a specially designed questionnaire and Nottingham Extended ADL Index. The survey was voluntary, anonymous and self-administered.In our study the highest scores on the NEADL scale were achieved by HMSN patients, and the lowest by patients with SMA3. The research revealed statistically significant differences between all the groups in the total number of points achieved on NEADL scale.The study revealed that for most respondents the most difficult tasks were those in the area of ‘mobility’. It is consistent with reports in the literature, which confirm that out of the slowly progressive neuromuscular diseases included in this research, SMA3 is a disease leading to the biggest limitations in performing the activities of everyday life.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Rumyana Kuzmanova, Irina Stefanova, Katerina Stambolieva IntroductionThe absence of patient's cooperation when it comes to his/her treatment (“noncompliance”) is typical to chronic diseases and it is significant problem in medical practice. The term “compliance” means patient's capability of strictly adhering to the recommendations concerning the prescribed treatment. The noncompliance with drug regime is frequent case in patients with epilepsy, it is related to increased risk of epileptic seizures’ occurrence and other undesired consequences, including increased costs in the healthcare area.ObjectiveThe objective of our research is assessing the interconnection between compliance with the treatment and social-demographic and clinical factors in patients with epilepsy.Contingent and methodsThe research covers 131 consecutively included patients with epilepsy of various social-demographic and clinical characteristics. We have utilized analysis of the medical documentation, anamnesis, study of the somatic and neurological status, self-assessment scales and statistical methods.ResultsWe established statistically significant positive correlations between the number of patients with poor compliance and the absence of professional/educational occupation, frequency of epileptic seizures, number of the antiepileptic drugs taken during the present and past treatment, the simultaneous presence of poor control of epileptic seizures and adverse drug events being the reason behind the modification of the previous treatment.ConclusionPatient's poor compliance, the great frequency of seizures, the higher number of antiepileptic drugs and the adverse drug reactions have negative impact on the course of the epileptic disease. The improved compliance results in optimizing the antiepileptic treatment, improving patients’ condition and significantly cutting down costs incurred in the healthcare area.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Vladimir Rendevski, Boris Aleksovski, Dragan Stojanov, Vasko Aleksovski, Ana Mihajlovska Rendevska, Milena Kolevska, Kiro Stojanoski, Icko Gjorgoski ObjectiveWe aimed to evaluate the prognostic values, contribution and interactions of the peripheral blood plasma glutamate and tumor-necrosis factor-α (TNF-α) levels toward the formation of the perifocal edema in patients with intracerebral hemorrhage (ICH).MethodsFifty patients with ICH and fifty healthy controls were included in the study. The peripheral markers were detected by high-sensitivity ELISA.ResultsA highly significant differences in plasma glutamate and TNF-α levels with good separation of their values was detected between patients and healthy controls. The two variables correlated with the severity of the symptoms and the initial volume of the ICH at admission. Both peripheral glutamate and TNF-α levels at admission were estimated as significant predictors for the formation of the perifocal edema five days after ICH; nevertheless, it was shown that they independently contribute to the development of the edema, without effects of interaction and regardless the localization of the ICH.ConclusionsOur results support the idea for the significance of glutamate and TNF-α as peripheral markers for excitotoxicity and inflammation in ICH patients. The developed multiple regression model for prediction of the development of the edema could be beneficial in decision making between conservative treatment and surgical intervention in the clinical practice.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Elif Gökçal, Elvin Niftaliyev, Tuğçe Özdemir, Mehmet Kolukısa, Talip Asil Arterial calcification (AC), a frequent finding on imaging studies, has been reported as a risk factor for ischemic stroke. However few studies have explored the association of AC with etiological subtypes and prognostic implications. The purpose of this study was to investigate the association of AC with demograhics, risk factors and etiological subtypes and to determine whether it predicts stroke recurrence and functional outcome in patients with acute brainstem ischemic stroke. We analyzed our database consisting of patients who were diagnosed as acute brainstem ischemic stroke admitted within 24 hours of onset. Etiological classification of stroke was made based on The Trial of ORG in Acute Stroke Treatment (TOAST) Classification. AC in vertebral and basilar arteries were assessed from baseline brain CT. AC degree was categorized according to calcification along the circumference in the densest calcified segment of each vessel (0 point if no calcification in any of the CT slices, 1 point if calcification

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Iwona Obuchowska, Grzegorz Turek, Jan Kochanowicz, Andrzej Lewszuk, Zofia Mariak, Zenon Mariak Late ocular manifestations of aneurysmal subarachnoid hemorrhage (SAH) have not been previously investigated except for one study which demonstrated that one half of patients subjected to aneurysm clipping suffer from symptoms of visual pathway impairment. We assessed ophthalmological status of patients after 1–4.5 years from SAH and aneurysm embolization to identify predictors of damage to the visual pathways. Complete ophthalmological examination, static perimetry, and visual evoked potentials (VEPs) were performed in 74 patients (26 men, 48 women, aged 19–76 years), who constituted a consecutive sample of 129 patients treated with aneurysm embolization in the years 2008–2010. The following independent variables: sex, age, time from SAH to embolization, size and site of aneurysm, score in Glasgow Coma Scale, Glasgow Outcome Scale, Hunt-Hess and Fisher scales were subject to univariate and multivariate statistical analyses to study their influence on the ocular outcome. 40 patients (54%) demonstrated visual field defects appearing as multiple peripheral foci and constricted field, affecting both eyes. Among these subjects, 12 patients had severe defects in the visual field, 20 had deterioration in VEPs, and 9 had decreased visual acuity. Older age and high score in Hunt-Hess and Fisher scales were identified as predictors for visual field defects and disturbances in VEPs. More than half of the survivors of SAH and aneurysm embolization suffer from a permanent defect in visual function. Damage of visual pathway correlates with severity of SAH and older age of patients.

Abstract: Publication date: March 2018Source: Neurologia i Neurochirurgia Polska, Volume 52, Issue 2Author(s): Agnieszka Madej-Pilarczyk, Michał Marchel, Karolina Ochman, Joanna Cegielska, Roman Steckiewicz Mild skeletal muscle symptoms might be accompanied with severe cardiac disease, sometimes indicating a serious inherited disorder. Very often it is a cardiologist who refers a patient with cardiomyopathy and/or cardiac arrhythmia and discrete muscle disease for neurological consultation, which helps to establish a proper diagnosis. Here we present three families in which a diagnosis of skeletal muscle laminopathy was made after careful examination of the members, who presented with cardiac arrhythmia and/or heart failure and a mild skeletal muscle disease, which together with positive family history allowed to direct the molecular diagnostics and then provide appropriate treatment and counseling.

Abstract: Publication date: Available online 12 February 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Elżbieta Mirek, Magdalena Filip, Wiesław Chwała, Jadwiga Szymura, Szymon Pasiut, Krzysztof Banaszkiewicz, Monika Rudzińska Bar, Andrzej Szczudlik ObjectiveThere is no existing standard, evidence-based, scientific model for motor ability improvement in Huntington's Disease (HD) patients aimed at maintaining independent gait for as long as possible, or performing activities of daily living, the effectiveness of which would be supported by the results of studies using objective research tools. Under these circumstances, the aim of this study was to analyze the influence of motor ability rehabilitation on the spatial-temporal parameters of gait in HD patients.DesignIt was an experimental trial. The studied group consisted of 30 patients (17 women and 13 men) with HD. In hospital conditions, the patients participated in the 3-week motor ability l rehabilitation programme tailored to individual needs. The study group was tested using the Vicon 250 three-dimensional gait analysis system before and after the physical exercise programme.ResultsWalking speed after therapy increased for the left lower limb from 1.06 (SD 0.24) [m/s] to 1.21 (SD 0.23) [m/s], and for the right lower limb from 1.07 (SD 0.25) [m/s] to 1.20 (SD 0.25) [m/s]. The cycle length increased after the applied therapy for the left lower limb from 1.17 (SD 0.20) [m] to 1.23 (SD 0.19) [m].ConclusionThe three-week motor ability rehabilitation programme positively influences spatial-temporal gait parameters in HD patients.

Abstract: Publication date: Available online 6 February 2018Source: Neurologia i Neurochirurgia PolskaAuthor(s): Mehmet Hakan Seyithanoğlu, Serkan Kitiş, Ömer Faruk Özer, Abdurrahim Koçyiğit, Tolga Dündar, Meliha Gündağ Papaker, Anas Abdallah BackgroundThe relationship between radiological degeneration criteria on lumbar magnetic resonance imaging (MRI) and both the keratan sulfate (KS) and chondroitin sulfate (ChS) levels was examined in disc material taken from patients undergoing lumbar disc herniation (LDH) surgery. To examine whether the biochemical and radiological degeneration criteria testing the reliability of radiological degeneration findings agreed and to evaluate the contribution of the KS/ChS ratio to disc form (protruding or extruding).MethodsThis was a prospective experimental cohort study. Using enzyme-linked immunosorbent assay, KS and ChS levels were measured in the degenerate nucleus pulposus taken from 71 patients with a diagnosis of LDH who underwent surgery. The degeneration levels and disc form (protruding or extruding) were determined according to the Pfirrmann five-stage grading system on preoperative T2-weighted lumbar MRIs. According to the Pfirrmann system, 28 patients were grade III and 43 were grade IV. The relationship between radiological criteria and the KS/ChS ratio was statistically evaluated.ResultsThe KS levels (p = 0.046) and the KS/ChS ratio (p = 0.001) were significantly higher in grade IV patients than in grade III patients. However, there was no difference between the KS and ChS levels and the KS/ChS ratio when patients were classified as protruding or extruding according to their disc structure. Disc structure and biochemical degeneration indicators were not correlated.ConclusionsThe KS level and the KS/ChS ratio were high in patients with marked radiological degeneration on lumbar MRI, demonstrating the sensitivity and reliability of the Pfirrmann five-stage grading system for showing radiological degeneration.